2015
DOI: 10.1016/j.jns.2015.05.003
|View full text |Cite
|
Sign up to set email alerts
|

JC virus granule cell neuronopathy: A cause of infectious cerebellar degeneration

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
4
1

Citation Types

1
20
0

Year Published

2015
2015
2022
2022

Publication Types

Select...
6
1

Relationship

0
7

Authors

Journals

citations
Cited by 24 publications
(21 citation statements)
references
References 27 publications
1
20
0
Order By: Relevance
“…PMLprogression heterogeneity has been described since the first cases and long-term survival (10%) is known since 1988 [20]. Albeit remarkable, our patient's prolonged survival is consistent with other JCV-GCN observations [8,9,16]. Whether JCV-GCN has a better prognosis than other PML forms is unclear.…”
Section: Introductionsupporting
confidence: 61%
See 1 more Smart Citation
“…PMLprogression heterogeneity has been described since the first cases and long-term survival (10%) is known since 1988 [20]. Albeit remarkable, our patient's prolonged survival is consistent with other JCV-GCN observations [8,9,16]. Whether JCV-GCN has a better prognosis than other PML forms is unclear.…”
Section: Introductionsupporting
confidence: 61%
“…JCV-GCN is sometimes associated with T2-weighted/fluid-attenuated inversion recovery (FLAIR) hyperintense white matter abnormalities typical of PML but is a distinct clinical entity [1]. Only 24 cases of HIVassociated JCV-GCN, including our patient, have been reported [9][10][11][12][13][14][15][16].…”
Section: Introductionmentioning
confidence: 99%
“…JCV affects the neurons in the cerebellum when there is a mutation of the VP1 gene coding for the major capsid protein, which changes the viral tropism from glial cells to cerebellar granule cells. 2,4,10,14 This clinical syndrome is called granule cell neuronopathy, and the clinical manifestations include balance instability and impaired coordination with or without slurring of speech or abnormal eye movements. This atypical presentation can be diagnostically challenging, delaying testing for JCV-associated disease and the initiation of immune reconstitution.…”
Section: Discussionmentioning
confidence: 99%
“…[1][2][3] Normally, cellular immunity keeps the virus quiescent where it is harbored (in the urinary tract, bone marrow, lymphoid tissue, and possibly the central nervous system). 2,4,5 However, patients with abnormalities in cell-mediated immunity are vulnerable to viral reactivation, and replication occurs in glial cells (predominantly oligodendrocytes) and neurons of the cerebrum and granular cell layer of the cerebellum. This leads to demyelination and neuronal death, causing mortality within 2 to 15 months of diagnosis; however, death usually occurs within 2 months for 90% of patients who also have a hematologic malignancy.…”
Section: Introductionmentioning
confidence: 99%
See 1 more Smart Citation